Síndrome de Stewart-Treves no membro inferior com apresentação exuberante: relato de caso / Profuse Stewart-Treves syndrome in the right leg: a case report

A Síndrome de Stewart-Treves é uma rara condição onde um angiossarcoma se desenvolve sobre área de linfedema crônico. Afeta mais frequentemente os membros superiores e representa 5% dos casos de angiossarcoma. Apesar de ser mais comum em mulheres submetidas a mastectomia radical e esvaziamento axilar por neoplasia de mama, pode ocorrer a partir de linfedema de outras etiologias. Clinicamente as lesões são caracterizadas por nódulos únicos ou múltiplos de cor avermelhada ou azulada no estágio inicial e, em estágio avançado, podem evoluir para massas hemorrágicas e até gangrena. O prognóstico é ruim e a conduta cirúrgica com excisão ampla ou amputação oferecem uma maior chance de sobrevida. Relata-se um caso de Síndrome de Stewart-Treves na perna direita, apresentando-se de forma exuberante e com acometimento de linfonodos pélvicos e abdominais.

Stewart-Treves syndrome is a rare condition characterized by the development of an angiosarcoma at sites of chronic lymphedema. It most often affects the upper limbs and accounts for 5% of cases of angiosarcoma. Although the syndrome is more common in women undergoing radical mastectomy and axillary dissection for breast cancer, it may result from lymphedema of other etiologies. Clinically, the lesions are characterized by single or multiple bluish-red nodules in the early stage and may progress to hemorrhagic masses and even gangrene in advanced stages. The prognosis is poor, and surgical management with wide excision or amputation offers a greater chance of survival. We report a case of profuse Stewart-Treves syndrome in the right leg, with involvement of pelvic and abdominal lymph nodes.

Primary Angiosarcoma of the Breast: A Case Report

Primary angiosarcoma of the breast is extremely rare and is observed in 0.0005% to 0.05% of primary breast tumor cases. The diagnosis of this tumor is difficult due to its undefined characteristics. Radiologic findings are often nonspecific and appear completely normal in one-third of patients with primary angiosarcomas. The prognosis is usually poor, and the treatment choices include mastectomy or wide excision. Radiotherapy and chemotherapy produce varying results. We report a patient with primary angiosarcoma of the breast to further our understanding of the characteristics of this tumor and facilitate the correct diagnosis of breast angiosarcoma.

Cutaneous angiosarcoma: report of three different and typical cases admitted in a unique dermatology clinic

Abstract: Angiosarcoma is a rare and aggressive tumor with high rates of metastasis and relapse. It shows a particular predilection for the skin and superficial soft tissues. We report three distinct and typical cases of angiosarcoma that were diagnosed in a single dermatology clinic over the course of less than a year: i) Angiosarcoma in lower limb affected by chronic lymphedema, featuring Stewart-Treves syndrome; ii) a case of the most common type of angiosarcoma loated in the scalp and face of elderly man and; iii) a skin Angiosarcoma in previously irradiated breast. All lesions presented characteristic histopathological findings: irregular vascular proliferation that dissects the collagen bundles with atypical endothelial nuclei projection toward the lumen.

Stewart-Treves Syndrome of the Lower Extremity

Stewart-Treves syndrome is a rare cutaneous angiosarcoma that develops in long-standing chronic lymphedema. Though most commonly this angiosarcoma is a result of post mastectomy lymphoedema, it also develops in Milroy disease, idiopathic, congenital, traumatic and filarial lymphoedema. Despite the rarity of this syndrome and its poor prognosis, early diagnosis associated with radical surgery can provide improved survival. We report a case of angiosarcoma in the lower limb in a patient with chronic lymphedema associated with history of repeated erysipela episodes.

Stewart Treves Syndrome

Stewart-Treves Syndrome is characterized by the presence of lymphangiosarcoma on limb extremities. Rare, it occurs in 0.5% of patients who have undergone radical mastectomy with axillary node dissection. The main cause is chronic lymphedema with endothelial and lymphatic differentiation, with no direct relationship to breast cancer. Seven years after a radical right-side mastectomy with lymph node dissection and adjuvant therapy, the patient developed a lesion on her right arm. The dermatological examination revealed an erythematous nodule with bleeding surface on chronic right forearm lymphedema. After the biopsy, a lymphangiosarcoma on chronic lymphedema was diagnosed. Infrequent, this syndrome is relevant because of its associated mortality. Early diagnosis is important to improve survival and reduce complications.

Stewart-Treves syndrome angiosarcoma expresses phenotypes of both blood and lymphatic capillaries / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The development of angiosarcoma in oedematous tissue is referred to as Stewart-Treves syndrome (STS). This rare and fatal complication is associated with chronic post mastectomy lymphoedema and radiotherapy for breast cancer. Angiosarcoma spread is facilitated by the formation of blood vessels (angiogenesis) and lymph vessels (lymphangiogenesis). In the future antiangiogenic therapy may improve the poor outcome of current treatments. There was evidence that blocking the angiogenenesis would inhibit progression of angiosarcoma. It seems reasonable to hypothesize that blocking the lymphangiogenesis may yield similar results. Although angiosarcomas commonly derive from blood vessels, in case of STS angiosarcomas chronic lymphoedema may suggest its lymphatic origin. The goal of this study was to visualize interstitial space and lymphatics in the central and peripheral regions of STS angiosarcoma.</p><p><b>METHODS</b>On tissue samples obtained from STS angiosarcoma we have performed: first colour stereoscopic lymphography to visualise the morphology of lymphatic vessels and extracellular spaces, second immunohistochemical staining specific for lymphatic vessels endothelium (LYVE-1) and blood endothelial cells (CD31, factor VIII) and prolymphangiogenic vascular endothelial growth factor (VEGF-C) for precise identification of lymphatic endothelia. STS angiosarcoma morphology was assessed by comparison of pictures obtained on lymphography, microscopy and confocal microscopy.</p><p><b>RESULTS</b>STS angiosarcomas present heterogenous morphology with areas dominated by hemangiosarcoma and lymphangiosarcoma structures. STS angiosarcoma expressed phenotypes of both blood and lymphatic endothelia. LYVE-1 and VEGF-C is expressed by STS angiosarcoma and may be used to discriminate tumour differentiation. Morphology of lymphatic vessels and spaces in the tumour suggest absence of their normal lymphatic function.</p><p><b>CONCLUSIONS</b>Our results confirmed both hemangio- and lymphangiogenic origin of STS angiosarcoma. Expression of VEGF-C makes STS angiosarcoma a good candidate for targeted antilymphangiogenic therapy. However, morphology of intratumoral lymphatics on colour lymphography suggested their impaired function, which can hamper drug distribution.</p>

Síndrome de Stewart-Treves: Caso clínico / Stewart-Treves syndrome: Report of one case

Angiosarcomas are malignant tumors derived from the endothelium of blood vessel (hemangiosarcomas) or lymph vessels (lymphangiosarcomas). Lymph edema of the limbs is considered secondary when extrinsic injuries are observed and primary when these injuries are not present. Stewart-Treves syndrome or a Lymphangiosarcoma, developed over a chronic lymph edema, is a rare complication described in mastectomized patients but it can be observed in lymph edemas located elsewhere. It appears as nodular skin lesions that grow, multiply quickly and frequently metastasize. We report a 40-year-old mole with an angiosarcoma associated with primary chronic lower limb lymph edema. The patient consulted for a history of weight loss and malaise and appearance of violaceous lesions over the zone of lymph edema and inguinal lymph node involvement. A CT scan showed bilateral lung lesions and enlargement of inguinal and iliac lymph nodes. A biopsy of one of the skin lesions disclosed an angiosarcoma, diagnosis that was confirmed with immuno-histochemistry Chemotherapy was started but the patient died five months after the diagnosis.

Stewart-Treves syndrome: case report

In 1948 Stewart and Treves described a syndrome related to the association between lymphangiosarcoma and chronic lymphedema due to radical mastectomy and radiotherapy. Currently, literature data reveals around 400 published cases. However, this pathology is becoming each time rarer due to the growing indication of conservative breast surgery and sentinel lymphonode research, thus reducing the need of axillary lymph node dissection with subsequent lymphedema. Described will be the case of a woman that developed angiosarcoma in shoulder 17 years after mastectomy with adjuvant radiotherapy. Stewart-Treves syndrome is related to the rise of angiosarcoma in patients with chronic lymphedema. It is currently treated as a rare disease. We describe the case of a woman who developed angiosarcoma in shoulder 17 years after mastectomy with adjuvant radiotherapy.

Síndrome de Stewart Treves: relato de caso / Stewart-Treves syndrome: case report

Apresentamos um caso de linfoangiossarcoma de membro superior esquerdo, em paciente de 52 anos, previamente submetida à mastectomia radical modificada à esquerda, seguida de radioterapia devido ao carcinoma de mama 6 anos antes. Esta rara síndrome está associada a linfedema crônico, após mastectomia radical, seguida de radioterapia. Aproximadamente 400 casos foram relatados na literatura. A ocorrência infreqüente dessa doença e a aparência inócua do tumor, freqüentemente levam a retardo no diagnóstico e tratamento. No caso dessa paciente, o diagnóstico foi obtido por meio de biópsia incisional da lesäo, com confirmaçäo pela imuno-histoquímica, utilizando marcadores de células endoteliais, anticorpo anti-CD31, vimentina e actina muscular. A paciente foi submetida à amputaçäo do membro superior, e encontra-se em seguimento sem recorrência local ou à distância há 18 meses

Acquired Lymphangioma(Lymphangiectasis) in the Patient with Congenital Lymphedema / 대한피부과학회지

Lymphangioma, occuring as a rare long-term complication of chronic lymphedema, has been described in association with scarring process including recurrent infections, radiotherapy, scrofuloderma, scleroderma, keloid, and tumors. Only one case has been reported in the patient with congenital lymphedema in English literature. The case discussed in this report developed 40 years after following congenital lymphedema. Both this condition and lymphangiosarcoma, its malignant counterpart, may arise in lymphedematous extremities regardless of the etiology of lymphatic obstruction.

Linfangiossarcoma pós-mastectomia: síndrome de Stewart-Treves; relato de um caso e revisäo da literatura

Os autores relatam um caso de linfangiossarcoma pós-mastectomia (síndrome de Stewart-Treves). Trata-se de complicaçäo rara e extremamente grave do linfedema crônico. Após revisäo da literatura, concluem que todas as formas terapêuticas, incluindo cirurgia, radioterapia e quimioterapia, säo insatisfatórias. Para melhorar a sobrevida, é fundamental o diagnóstico precoce através de biópsia em todos os casos de lesäo suspeita. A prevençäo é também muito importante, através da profilaxia do linfedema pós-operatório e do tratamento adequado quando este ocorre

Radiation Treatment of Postmastectomy Lymphangiosarcoma / 대한치료방사선과학회지

Since the entity of postmastectomy lymphangiosarcoma was first reported by Stewart and Traves in 1948, postmastectomy lymphangiosarcoma has become a well recognized, uncommon malignant tumor which occurs in the upper extremity following mastectomy for mammary carcinoma. The postmastectomy lymphangiosarcoma occurred at an average age of 63.9 years and at an average of 10 years and 3 months following mastectomy. The lymphangiosarcoma raised from blood and lymphatic vessel. The histologic appearance has been observed edematous dermis and dilated lymphatics lining with malignant cells. Most authors recommend radical amputation for treatment, either shoulder disarticulation or forequarter amputation. Other modalities of treatment including radiotherapy were considered as ineffective. The present report provides a case of the regression of postmastectomy lymphangiosarcoma with chronic lymphedima by external irradiation. Radiation therapy was used as primary therapy. Total tumor dose of 6500 cgy in 9 weeks was delivered using 6 MV x-ray and 8 MeV electron.

Linfangiosarcoma de pierna en un miembro elefantiásico / Leg lymphangiosarcoma in an elephantiasis limb

Los autores presentan un caso de linfangiosarcoma asentando en un miembro con linfedema crónico, de evolución rápidamente progresiva, en el cual no se pudo realizar ningún gesto terapéutico. Se hace un análisis de estos tumores angioformadores, que constituyen una rareza

Sindrome de Stewart-Treves. / StewartTreves syndrome

Dos casos de angiosarcoma post-mastectomia (sindrome de Stewart-Treves) son presentados. El tiempo transcurrido entre la intervencion quirurgica y la aparicion del angiosarcoma fue de 21 anos en la primera paciente y de 9 anos en la segunda. La sobrevida fue escasa, a pesar del tratamiento implementado. Son realizados comentarios clinicos, histologicos y diagnosticos diferenciales

Sindrome de Stewart-Treves. / Stewart-Treves' syndrome

Se presentan dos casos de sindrome de Stewart-Treves y se describen sus aspectos clinicos e histopatologicos. Se realizaron estudios de inmunohistoquimica con metodo de peroxidasa antiperoxidasa para antigeno relacionado con el factor VII de la coagulacion como marcador de celulas endoteliales. Los mismos dieron positividad de dicho antigeno para sectores del tumor diferenciado hacia estructuras vasculares sanguineas, similar a lo observado para el hemangiosarcoma clasico, lo cual avalaria la histogenesis vascular sanguinea de la neoformacion

Los linfedemas cronicos primitivos. A proposito de dos casos no familiares. / Primary chronic lymphedema. Apropos of two nonfamiliar cases

Se presentan dos pacientes, uno de cada sexo, portadores de linfedemas primitivos no familiares. En uno de ellos el proceso es bilateral, y como hecho llamativo se asocia a un sindrome de Apert. En el otro es unilateral, destacandose su complicacion con un angiosarcoma de Stewart-Treves. De acuerdo a la edad de aparicion, ambos serian linfedemas de tipo precoz (no congenito y anterior a los 35 anos de edad). Sus caracteristicas clinicas son tipicas y los hallazgos histopatologicos coincidentes con los de la bibliografia. Se los clasifica, segun su posibilidad de asociacion,en: solitarios,con asociaciones diversas y sindromicos (segun que sus asociaciones sean ocasionales o frecuentes).En este ultimo grupo se ubica a los sindromes de Bonnevie-Ullrich, de las unas amarillas, linfedema-distiquiasis y linfedema-ptosis palpebral.Los estudios de inmunidad celular efectuados en un paciente con DNCB no son concordantes con los de otros autores

Linfangiosarcomas postmastectomia. / Post-mastectomy lymphangiosarcomas

Se describen 4 casos de linfagiosarcoma postmastectomia con referencia especial a los aspectos clinicos y patologicos, enfatizando la importancia del diagnostico precoz y urgente tratamiento quirurgico, en razon de su alta malignidad y rapida diseminacion. Se lo observa cada vez con menos frecuencia. Estudios estadisticos indican una incidencia de 7 casos de cada 1.000 pacientes operadas por cancer de la mama. Esta baja incidencia es debida en parte a las medidas actuales de prevencion del linfoedema y tambien porque ahora se opera en estudios mas tempranos. Histologicamente el linfangiosarcoma postmastectomia es un tumor indiferenciado, con gran proliferacion vascular que ha sido algumas veces clasificado como linfangiosarcoma y otras como angiosarcoma. Los muy pocos casos que sobreviven han sido tratados con cirugia radical (amputacion interescapulotoracica). El uso de otras tecnicas mas conservadoras y de la radioterapia han francasado. La quimioterapia recomendada en los ultimos anos no ha probado ser uti